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      Orthognathic surgery for patients with fibrous dysplasia involved with dentition

      case-report

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          Abstract

          Background

          Fibrous dysplasia (FD) is characterized by the replacement of normal bone by abnormal fibro-osseous connective tissue and typically treated with surgical contouring of the dysplastic bone. When dysplastic lesions involve occlusion, not only is surgical debulking needed, orthognathic surgery for correction of dentofacial deformity is mandatory. However, the long-term stability of osteotomized, dysplastic bone segments is a major concern because of insufficient screw-to-bone engagement during surgery and the risk of FD lesion re-growth.

          Case presentation

          This case report reviewed two patients with non-syndromic FD that presented with maxillary occlusal canting and facial asymmetry. Le Fort I osteotomy with recontouring of the dysplastic zygomaticomaxillary region had been performed. The stability of osseous segments were favorable. However, dysplastic, newly formed bone covered the previous plate fixation site and mild bony expansion was observed, which did not influence the facial profile. Including the current cases, 15 cases of orthognathic surgery for FD with dentition have been reported in the literature.

          Conclusion

          The results showed that osteotomy did not appear to significantly reduce the long-term stability of the initial fixation insufficiency of the screw to the dysplastic bone. However, based on our results and those of the others, long-term follow-up and monitoring are needed, even in cases where the osteotomized segment shows stable results.

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          Most cited references18

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          Craniofacial fibrous dysplasia of the fronto-orbital region: a case series and literature review.

          Craniofacial fibrous dysplais (CFD), involving the fronto-orbital region often is challenging to treat because of the proximity of neurovascular and ocular structures. This article presents the surgical experience with 6 patients. Six patients ranging in age from 7 to 23 years, who had undergone surgery for CFD of the orbital region, were retrospectively reviewed. All patients had progressive complaints of deformity, pain, paresthesia or visual disturbances. Surgery generally involved extensive tumor excision and immediate orbital reconstruction with autogenous bone grafts. Two patients also were reconstructed with resected and treated autogenous bone, which then was immediately reimplanted, using rigid fixation. Three patients also had intracranial microsurgical optic canal decompression. All patients received perioperative corticosteroids. Five patients experienced partial relief of their sensory and visual disturbances. These patients also felt that there had been improvement in facial appearance. One patient with very extensive circumferential optic canal involvement, and who underwent 2-wall decompression, suffered visual loss. Early surgery to address progressive sensory disturbances is recommended so as to avoid the hazards of late-stage decompression. The risks of surgery must be weighed against the benefits of achieving a more functional and aesthetic result. Long-term neuro-ophthalmologic monitoring is essential.
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            Fibrous dysplasia of bone: craniofacial and dental implications

            Fibrous dysplasia (FD) is a rare bone disease caused by postzygotic somatic activating mutations in the GNAS gene, which lead to constitutive activation of adenylyl cyclase, and elevated levels of cyclic AMP, which act on downstream signaling pathways, and cause normal bone to be replaced with fibrous tissue and abnormal (woven) bone. The bone disease may occur in one bone (monostotic), multiple bones (polyostotic), or in combination with hyperfunctioning endocrinopathies and hyperpigmented skin lesions (in the setting of McCune-Albright Syndrome). FD is common in the craniofacial skeleton, causing significant dysmorphic features, bone pain, and dental anomalies. This review summarizes the pathophysiology, clinical findings and treatment of FD, with an emphasis on the craniofacial and oral manifestations of the disease.
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              The surgical management of fibrous dysplasia of bone

              The surgical management of Polyostotic Fibrous Dysplasia (FD) of bone is technically demanding. The most effective methods to manage the associated bone deformity remain unclear. The marked variation in the degree and pattern of bone involvement has made it difficult to acquire data to guide the surgeon’s approach to these patients. In light of the paucity of data, but need for guidance, recognized experts in the management of these patients came together at the National Institutes of Health in Bethesda, Maryland as part of an International meeting to address issues related to fibrous dysplasia of bone to discuss and refine their recommendations regarding the surgical indications and preferred methods for the management of these challenging patients. The specific challenges, recommended approaches, and “lessons learned” are presented in hopes that surgeons faced with typical deformities can be guided in the surgical reconstruction of both children and adults with FD.
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                Author and article information

                Contributors
                ishu2691@gmail.com
                jypaeng@gmail.com
                dentalchoi@knu.ac.kr
                hishin@knu.ac.kr
                st0907@knu.ac.kr
                +82-53-600-7574 , kwondk@knu.ac.kr
                Journal
                Maxillofac Plast Reconstr Surg
                Maxillofac Plast Reconstr Surg
                Maxillofacial Plastic and Reconstructive Surgery
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                2288-8101
                2288-8586
                3 December 2018
                3 December 2018
                December 2018
                : 40
                : 1
                : 37
                Affiliations
                [1 ]ISNI 0000 0001 0661 1556, GRID grid.258803.4, Department of Oral and Maxillofacial Surgery, School of Dentistry, , Kyungpook National University, ; 2177 Dalgubeol-daero, Jung-gu, Daegu, 41940 Republic of Korea
                [2 ]ISNI 0000 0001 2181 989X, GRID grid.264381.a, Department of Oral and Maxillofacial Surgery, Samsung Medical Center, , Sungkyunkwan University School of Medicine, ; Seoul, Republic of Korea
                [3 ]ISNI 0000 0001 0661 1556, GRID grid.258803.4, Department of Oral Pathology, School of Dentistry, Institute for Hard Tissue and Bio-tooth Regeneration, , Kyungpook National University, ; 2177 Dalgubeol-daero, Jung-gu, Daegu, 41940 Republic of Korea
                Author information
                http://orcid.org/0000-0003-2799-0510
                Article
                176
                10.1186/s40902-018-0176-y
                6275155
                907d600c-a362-4d43-9677-116cd9c9f3e2
                © The Author(s). 2018

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                : 9 October 2018
                : 19 October 2018
                Funding
                Funded by: National Research Foundation of Korea (KR)
                Award ID: 2014RA1008141
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2018

                fibrous dysplasia,orthognathic surgery,le fort i,osteotomy,rigid fixation

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